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Early-onset nonprogressive chorea.

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absence of proof-in this case of a declining rate-does not
constitute proof of absence. Moreover, a declining rate of
later rebleeding would be incompatible with a simple hypertensive cause for hemorrhage, as one would expect a rebleed
rate which would increase with time if such an origin were
the case.
T h e sixth month to one year rate is, in fact, plotted at one
year. There were no rebleeds in the tenth year, hut we
attributed this to the diminishing sample size with time and
statistical variation. Subsequent follow-up, however, has
revealed rebleed in the tenth year in 1 ( 2 % ) of the ‘19
patients still being followed at the time of the original study
(197 1).Moreover, in the eleventh pear 3 patients rebled out
of 65 patients at risk, a 4.69: rate for the eleventh year.
Similar calculations produce 1.7, 2.3 and 3.5% for the
twelfth, thirteenth, and fourteenth years, respectively.
In answer to D r McFarland’s query concerning the definity of proof of rebleeding, absolute proof was found in 45
patients. Following a subarachnoid hemorrhage proved by
lumbar puncture, 17 patients had arteriography that revealed enlargement of their previously defined aneurysm.
An additional 17 patients had postmortem examination
demonstrating rupture o f t h e previously identified aneurysm
as the cause of their death. There remained 11 patients
whose late rehemorrhage was proved by lumbar puncture
and whose histriry was compatible with a rebleed from the
previously documenreti aneurysm.
N o t reported in our article, hut to be presented i n a laterstudy, are several patients whose s u b s q u e n t subarachnoid
hemorrhage was secondary to nonaneurysmal causes as well
as 1 case of hypertensive encephalopathy. O n e patient had a
cerebellar hemorrhage twenty years after rupture of his
W e believe D r McFarland’s statement that “rebleeds from
the offending aneurysm are extremely rare 40 days following the last episode” is incorrect. Not only does our study
show the contrary, but previous studies cited in our article
support the fact that late rebleeding is an event which
clinicians must recognize in an unbiased way. T h e major
point of our study was to assess the risk from late hemorto previous
rhage, and the major conclusion-contrary
attitudes-is that the risks are not negligible. Moreover, the
recently reported cooperative study [ 11 cited by D r McFarland revealed that of 111 patients survivingto six months, 14
(13%)) experienced a h a 1 late rebleeding episode over a
six and one-half year follow-up. Thus, the average yearly
fatal rebleed rate was 2%, identical to that in our study.
Aneurysmal subarachnoid hemorrhage must therefore be
recognized as not just an acute illness, but also a chronic
process. Consequently, as we stated in our article, any
treatment modality, whether nreclical or szdrgicuf, must “ n o
longer deal with the short-term prognosis.”
Nonprogressive Chorea
Kamal Sad jadpour, MD,
and R. Stephen Amato, M D , P h D
Damasio et al (Ann Neurol 1:602-603, 1977) report a family
in which a boy and a girl, among 4 sibs, had the picture of
nonprogressive chorea of early onset. The authors failed to
mention the extensive family we described in 1073 [l]. We
were able to trace the disorder through five generations and
examine affected persons from three generations. In that
family, 49 individuals in the kindred were at risk for the
condition and 22 were affected. There was 1 instance of
male-to-male transmission.
From these datawe inferred that the disease is inherited as
an autosomal dominant condition with almost complete
penetrance, although with variable clinical expression.
1. Sadjadpour K, Amaro RS: Hereditary nonprogressive chorea of
early onset-a new entity?, in Barbeau A , Chase TN, Paulson
GW ieds): Advances in Neurology: Vol 1. Huntington’s chorea,
1872-1072. New York, Raven Press. 1973. pp 79-01
From the Division of Nrurusciences, Midland Hospital Center,
Midland. MI 48640.
Editor’s Note
T h e oversight of D r Sadjadpour’s communication by both
authors and editors reflects the frequent fate of manuscripts
published in nonindexed symposia and not subject to competitive editorial review.
1. Nibbelink DW, Torner JC, Henderson WG: Intracranial
aneurysms and subarachnoid hemorrhage: report o n a randomized treatment study: IV-A. Regulated bed rest. Stroke
8:202-218. 1977
Annals of Neurology
Vol 2
No S
November 1977
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nonprogressive, chorea, early, onset
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